BACKGROUND Hemolysis is a reported side effect of intravenous immunoglobulin (IVIG) therapy in adults, but pediatric data are scarce. We determined the frequency of IVIG‐associated hemolysis in patients with Kawasaki disease (KD) and characterized risk factors for hemolysis. We hypothesized that hemolysis is more common in children with KD than adults with other disorders, and hemolysis risk is related to IVIG dose and degree of inflammation. STUDY DESIGN AND METHODS This was an 8‐year, single‐center, retrospective cohort study. A total of 419 KD patients were identified; 123 had pre‐ and post‐treatment complete blood counts allowing for assessment of anemia. Hemolytic anemia was defined as decrease in hemoglobin after IVIG greater than 1 g/dL with immunohematologic or biochemical studies supporting hemolysis. RESULTS 123 patients were stratified as having hemolysis (n = 18, 15%) or nonhemolysis (n = 105, 85%). Patients with hemolysis were more likely to have complete versus incomplete KD (65% vs. 39%, p = 0.04) and refractory versus nonrefractory course (78% vs. 16%, p < 0.001). Patients receiving 4 g/kg versus 2 g/kg IVIG were more likely to hemolyze (89% vs. 34%, p < 0.001). Patients with hemolysis had mostly non‐O blood group (94%), positive direct antiglobulin tests (89%), and positive eluates (72%). Two‐thirds of patients with hemolysis required RBC transfusion. CONCLUSIONS Hemolysis occurred in 15% of KD patients evaluated for anemia and is strongly associated with high‐dose (4 g/kg) IVIG. KD patients receiving high‐dose IVIG should have close hematologic monitoring to identify hemolysis.
African individuals harbor molecular RH variants, which permit alloantibody formation to high-prevalence Rh antigens after transfusions. Genotyping identifies such RH variants, which are often missed by serologic blood group typing. Comprehensive molecular blood group analysis using 3 genotyping platforms, nucleotide sequencing, and serologic evaluation was performed on a 7-year-old African male with sickle cell disease who developed an "e-like" antibody shortly after initiating monthly red blood cell (RBC) transfusions for silent stroke. IntroductionRed blood cell (RBC) transfusion is a common treatment for acute sickle cell disease (SCD)-related complications, and for both primary prevention of stroke and secondary stroke prophylaxis. RBC alloimmunization occurs in 18% to 47% SCD patients, 1-3 compared with approximately 5% in thalassemia patients and 0.2% to 2.8% in the general population. 2 Alloantibodies to C, E, and K antigens are most commonly involved, leading many transfusion centers to supply Rh and K phenotype-matched RBCs for SCD patients. Despite this practice, alloimmunization continues to complicate their RBC transfusions.Reasons for the disproportionately high alloimmunization rates in SCD patients include in part disparate RBC antigens between donor and recipient due to clinically significant RH polymorphisms unrecognized by current serologic techniques. 2 Individuals of African origin frequently harbor variants of RHD and RHCE genes. The absence of high-prevalence Rh antigens, like hr S (Rh19), hr B (Rh31), and Hr B (RH34) in individuals homozygous for the variant predisposes them to Rh alloantibody formation after RBC transfusion, 4,5 making the long-term transfusion support difficult to manage.Hematopoetic stem cell transplantation (HSCT) has emerged as an alternative in many SCD patients who have severe disease, as a result of improved preparative regimens, graft sources, and reduction of HSCT-related side effects. 6 Despite these advances, guidelines on the selection of and timing of SCD who would maximally benefit from HSCT have not been fully defined. Current consensus on HSCT indications include stroke, recurrent severe acute chest syndrome, chronic unremitting vasoocclusive pain despite supportive care, or the inability to provide adequate supportive care such as chronic transfusion therapy or hydroxyurea. 7 Molecular technology has advanced our knowledge of RH polymorphisms, and its application has made RBC genotyping a clinically useful tool, often with superior accuracy to serologic phenotyping. 8 We present an informative patient history documenting the application of RBC genotyping in guiding both transfusion and HSCT donor selection strategy.
Our cases represent dose-dependent hemolysis caused by IVIG in association with severe anemia requiring transfusion with an average yearly incidence rate of 0.36%. Hemolysis is an underrecognized complication of IVIG administration. KD patients are at greater risk for anemia because of their lower baseline hemoglobin concentration, underlying acute inflammation, and oxygen requirements during acute illness.
BACKGROUND Alloantibodies against more than 50 non‐ABO blood group antigens have been implicated in hemolytic disease of the fetus and newborn (HDFN) and are expected to wane within weeks after delivery. Persistent anemia leads to the hypothesis of continued exposure to red blood cell (RBC) alloantibodies via breast milk, which has been shown in a murine model and suggested in rare case reports. CASE REPORT We report three cases of prolonged HDFN in two neonates with anti‐D HDFN and one with anti‐Jka HDFN. Patient 1 demonstrated 4+ anti‐D serologic testing beyond 2 months; therefore, antibody testing was performed on maternal breast milk. METHODS Maternal serum samples were tested for the presence of unexpected antibodies using standard Ortho gel card and 37 °C 60 minutes with anti‐human globulin (AHG) tube saline methods. Antibody titrations were performed using the standard 37 °C 60 minutes to AHG tube saline method. Fresh breast milk samples were tested using the standard 37 °C 60 minutes to AHG tube saline method for both unexpected antibodies and titration study. Fresh breast milk from an O‐positive, antibody‐negative donor was used as control for any reactivity that may have been due to milk solids or proteins alone. RESULTS Using a known methodology applied in a novel way to test breast milk for RBC alloantibodies, antibodies against fetal RBCs were identified in the maternal breast milk of three patients. CONCLUSION Maternal RBC alloantibodies are present in breast milk and may be clinically significant in patients with prolonged recovery from HDFN.
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